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Genetic counseling: Pancreatic Cancer
Pancreatic Cancer Pancreatic Cancer *Incidence is more than 29,000 new cases/year in US **Fifth leading cause of cancer death **4.1% five-year survival rate is lowest of all cancers **About 9-10% of pancreatic cancer is familial *Pancreas is a gland located in the abdomen between the stomach and the spine **Responsible for producing insulin, glucagon, and other hormones **Releases digestive juices through series of ducts leading to common bile duct *Risk factors for pancreatic cancer **Smoking - increases risk 2-3 times **Age - most cancers diagnosed between 60 and 80 years **Diabetes **Chronic pancreatitis ***Long-term inflammation of the pancreas ***Especially inherited form of the condition **Family history ***Risk increases by 4.7% if first degree relative has been diagnosed ***Increases by 7.3% if diagnosis was made before age 60 ***Increases by 12.3% if multiple primary cancers in family **Males and African-Americans may be at increased risk **Diets high in fat may increase risk Diagnosis and Surveillance Options *Possible symptoms **Pain in upper abdomen or upper back **Yellowing of skin and eyes, dark urine due to jaundice **Weakness **Loss of appetite **Nausea and vomiting **Weight loss *Physical exam **Look for signs of jaundice or changes in area near pancreas **Check for ascites (build-up of fluid in abdomen) *Lab tests **Blood, urine, and stool samples **Check for bilirubin that may be high if tumor blocking common bile duct prevents its passage *CT Scan **Produces detailed picture of abdomen **Can provide information about nature and location of tumors, resectability *Ultrasonography **Transabdominal ultrasound **Endoscopic ultrasound (EUS) ***Pass thin, illuminated optic instrument down mouth into stomach ***Ultrasound device at tip of endoscope takes images of pancreas and surrounding areas *Endoscopic Retrograde Cholangiopancreatography (ERCP) **Pass endoscope through mouth and stomach into first part of small intesting **Inject dye through catheter into ducts **Take X-rays to determine if ducts are narrowed or blocked by tumor *Percutaneous Transhepatic Cholangiography (PTC) **Dye injected through needle inserted through skin into liver **Dye moves freely through bile ducts unless blocked **X-rays can show if dye has moved through ducts *Biopsy **Fine-needle aspiration **During EUS or ERCP **Open abdomen during operation *Laparoscopy **Uses several small incisions and TV camera to visualize organs in abdomen **Usually only used if CT scan shows evidence of metastases Treatment *Pancreatic cancer is currently difficult to treat so participation in clinical trials is often recommended *Surgery **May be used alone or in combination with radiation or chemotherapy **Whipple procedure ***If tumor is in head of pancreas ***Remove head and part of small intestine, bile duct, and stomach **Distal pancreatectomy ***Surgeon removes body and tail of pancreas ***May also remove the spleen **Total pancreatectomy ***Remove entire pancreas ***Also remove part of small intestine and stomach, and all of common bile duct, gallbladder, spleen, and nearby lymph nodes **Create bypass or insert stent if tumor is blocking common bile duct or duodenum ***Relives jaundice and pain resulting from blockage ***Done if cancer cannot be completely removed surgically **Side effects of removal of all or part of pancreas ***Diet control and medication may help with food digestion ***Diarrhea, pain, cramping, and feelings of fullness are common *Radiation therapy and chemotherapy often used to destroy cancer cells Genetic Conditions Associated with Pancreatic Cancer *Familial Atypical Multiple Mole Melanoma (FAMMM) **Due to mutations in p16 tumor suppressor gene **TP16 (CKDN2) located at 9p21 **Affected individuals develop multiple nevi, atypical nevi, melanomas, and pancreatic cancer *Peutz-Jeghers Syndrome **Due to mutations in LKB1 (STK11) gene at 19p13.3 **Causes multiple hamartomaous polyps of GI tract and pigmented macules on lips, buccal mucosa, and digits **Increases risk to develop cancers ***In women: breast, pancreas, uterus, and ovary ***In men: colon, pancreas, lung, or testes *Hereditary Breast-Ovarian Cancer Syndrome **Due to mutations in BRCA2 tumor suppressor gene at 13q12 **Most common inherited predisposition to pancreatic cancer identified to date **Mutations in BRCA2 have been identified in individuals with no family history of breast or ovarian cancer **Estimated that carriers of 6174delT have 10-fold increased risk of developing pancreatic cancer **Increases risk for breast, ovarian, prostate, and colon cancers also *HNPCC **Due to mutations in mismatch repair genes, including MSH2 and MLH1 **Microsatellite instability has been identified in ~4% of pancreatic cancers **Should be considered in patients with family history of colon cancer **Increases risk for colon/rectum, endometrium, ovarian, urinary tract, stomach, and small intestine also *MEN1 **Due to mutations in menin tumor suppressor gene at 11q13 **Associated specifically with insulinomas (10%) or gastrinomas (40%) **Can cause tumors on parathyroid and pituitary glands also *Hereditary Pancreatitis **Due to mutations in cationic trypsinogen gene on 7q35 **Block inactivation of trypsin, resulting in autodigestion of pancrease **Increase in pancreatic cancer may be secondary to chronic injury and repair **Clinical testing available through University of Pittsburgh **Penetrance of mutations is only 80% Psychosocial Issues *Worry about the future - caring for themselves, family, etc *Financial concerns *Concerns about treatments and side effects *Concern about necessity of frequent hospital visits, home care, etc *Feelings of guilt, anxiety, fear surrounding potential diagnosis of hereditary syndrome Resources *The National Familial Pancreas Tumor Registry :Dr. Ralph H. Hruban, Director :Baltimore, MD 21231-2410 :Phone: (410) 955-9132 *Hereditary Pancreatitis Registry :Dr. Whitcomb or Dr. Lowenfels :The Midwest Multicenter Pancreatic Study Group :Phone: 888-PITT-DNA *National Cancer Institute :Phone: 1-800-4-CANCER :http://cancer.gov References *Hruban RH, et al. "Familial Pancreatic Cancer." Annals of Oncology (1999) 10:S69-S73. *"Pancreatic Cancer." Cancer Research UK. www.cancerresearchuk.org/aboutcancer *Schenk M, et al. "Familial Risk of Pancreatic Cancer." Journal of the National Cancer Institute (2001) 93:640-644. *"What you Need to Know About Cancer of the Pancreas. National Cancer Institute. www.nci.nih.gov/cancer Notes The information in this outline was last updated in June 2002. This material has been imported fom the wikibook "Genetic counseling"[ http://en.wikibooks.org/wiki/Genetic_counseling] under the GNU Free Documentation License.